OBJECTIVE: To provide information with the purpose of helping pediatricians to recognize the risk factors for the onset of severe early childhood caries, allowing for early intervention and thus avoiding this preventable disease and its consequences. SOURCES: Information was collected from scientific articles published on the databases SciELO, MEDLINE and PUBMED in last 25 years, as well as technical books and guidelines of international committees. The keywords used were: early childhood caries, severe early childhood caries, dental caries, and children. SUMMARY OF THE FINDINGS: Severe early childhood caries affects infants and children. It is infectious, has a multifactor etiology and fast development, starting soon after dental eruption. Due to the presence of local sociocultural risk factors, it must be regarded as a symptom of alteration in the child's health and lack of adequate care. Its manifestations include pain, abscesses and chewing difficulty, affecting the child's feeding and sleep. Furthermore, it also affects the child's general health, speech, and self-esteem. CONCLUSIONS: Preventive methods to avoid severe early childhood caries have been developed and they should be used as early as possible by means of prevention programs carried out in the community and with families. Professionals responsible for providing care to babies and children should be aware of cases at risk for caries and interfere in order to improve their patients' health.